Biliary Atresia: A Liver Transplant for Dominique
"The jaundice never went away"
Dominique Hill came home from the hospital jaundiced and within days her mother, Drunette, knew something was wrong. Tests confirmed biliary atresia, a rare disease of the liver and bile ducts. A series of medical procedures and frequent trips to Cincinnati Children's for monitoring and treatment made it possible for Dominique to live with this serious condition until age 13 when a liver transplant became the only solution. A successful transplant at Cincinnati Children's Hospital Medical Center in the spring of 2007 has given Dominique a second chance.
Drunette and Lawrence Hill live in Cincinnati. They have three daughters: Crystal, age 27; Jasmine, age 19; and Dominique, age 13. Since Dominique's birth, the Hills have been coping with the complications caused by biliary atresia. "At 6 weeks old her jaundice wasn't clearing up and her stool was clay-colored. Our pediatrician sent us to Cincinnati Children's for blood work on a Thursday and by Friday we were meeting with a surgeon to schedule diagnostic surgery."
The blood work results and a liver biopsy done at the Pediatric Liver Care Center at Cincinnati Children's confirmed the diagnosis. Suddenly, the family found themselves on a path with their newborn daughter that they never expected. Armed with a diagnosis, the family set about learning everything they could about liver dysfunction and treatment options. Drunette credits CIncinnati Children's web site for providing many of the answers she sought.
Early intervention
When a baby is born with biliary atresia, his or her bile ducts (a type of passageway inside and outside the liver) become narrowed due to inflammation and scarring. As the scarring increases, cirrhosis develops. Long term, the result is liver failure. At just six weeks of age Dominique had a Kasai procedure to open a duct and allow bile to drain from her liver. Nearly one-half of all infants who have had a Kasai procedure require liver transplantation before age 5. For Dominique it would be thirteen more years before a new liver would become critical. Still, the family would become frequent visitors to the Liver Care Center.
Emergency visits
For the first 13 years of her life, the family maintained a routine schedule of blood tests to monitor Dominique's liver function. At age seven, severe gastrointestinal (GI) bleeds from the rectum and stomach sent the family twice to the emergency room at Cincinnati Children's. Dominique was diagnosed with esophageal and fundal varices, a result of increased pressure in the blood vessels caused by liver scarring in association with biliary atresia. Dominique required a banding procedure to stop the bleeding. This was a serious development and one of the conditions that ultimately led to her placement on the national liver transplant waiting list.
Throughout her life Dominique has struggled with severe pruritis or itching. Commonly associated with a compromised liver, it can be aggravating and painful. At times, Dominique says, that her arms became so itchy she could not stop scratching. Her condition led to sores and rough patches, a condition that made Dominique feel self-conscious. Her skin became so thick and scarred that Dominique says it looked like it belonged to an elephant or alligator, not a little girl.
"Just Getting Through"
Social isolation is common for a child with a life-threatening or life-altering illness and Dominique was no exception. She was teased for the appearance of her skin, the hair that wouldn't grow (on her head and eyebrows) and her overall appearance as a child who is not well. Other children sometimes shunned her, worrying that they might somehow catch her disease or because they could only see what was different about her. "My best friend Amanda has been my friend since Kindergarten and sometimes she was the only one I could talk to about what I was going through," says Dominique.
But the family also received support from the community. Drunette recalls a fund-raiser held at her daughter's elementary school that raised money to help the family with medical bills and related expenses. "I've missed a lot of work," says Drunette. "It's hard to take time off without pay, but I'll do anything to be there for Dominique. Some days it's just a matter of getting through what comes next."
All in the Family
Around this time, Drunette joined the staff at Cincinnati Children's as a health unit coordinator. She recalls witnessing for the first time the incredible care and service provided to every child who comes to the hospital -- not just hers. "I saw for myself how phenomenal the nurses were," says Drunette. "I realized that it wasn't just our care team that was so good, it was everyone."
When their daughter was placed on the transplant list, the Hills once again scrambled to learn everything they could about what was next for their daughter. "I didn't know how the transplant list worked, but I'm someone who reads everything and does the research," says Drunette. The Liver Care Center's online information proved to be very useful to the Hills. There they could read up on symptoms and conditions and use the Liver Care Portal to monitor their daughter's lab results and progress following each visit to Cincinnati Children's.
Positive Numbers
Status on the waiting list for a new liver is determined by a mathematical formula based on the results of three blood tests. Before she turned 12 years old, Dominique's number was low, meaning that she was low-priority for a transplant. But this changed as she approached her teenage years. When an echocardiogram showed evidence of altered blood flow, Dominique qualified for extra points on the waiting list. Receiving a new liver became a priority.
The Phone Rings
When a child is on the liver transplant list, the family is given a pager so that as soon as a liver becomes available the family can be notified. On April 3, 2007, Drunette was on pager duty. "When I was first given the pager, I always thought I heard it going off," says Drunette. "So when I got a phone call, I didn't understand right away what they were saying." Instead of a page, Drunette's work line rang just as she was walking into her office. Accustomed to frequent visits to the Liver Care Center, she initially thought the care coordinator was suggesting a routine visit. Instead, Dominique was wanted at the hospital immediately for a liver transplant.
It wasn't until she hung up the phone that the impact of the call hit her. "Suddenly I had to sit down. My heart felt like it was going to beat out of my chest," Drunette says. Lawrence, who had just driven Dominique to school, turned back around, understanding immediately the seriousness of the phone call.
"Dominique started calling her friends, telling them, 'I'm going to get my new liver!'" says Drunette. While her parents felt the gravity of this sudden development, Dominique, who had been waiting for this moment her whole life, just felt ready.
An Amazing Ride
Dominique's surgery lasted just over four hours. For the family, the wait was excruciating. Much of their fear was alleviated by a stream of updates provided by Pastor Kevin Adams, MDiv, BCC, a hospital chaplain with the Liver Care Center. "He told us what was happening every step of the way. His support was awesome," says Drunette.
The morning after her transplant, Dominique was taken off the respirator. Her first question was, "When will I get to eat?" Drunette was surprised by how quickly her daughter came out of anesthesia. "She woke up and started asking questions, telling me what DVDs she wanted to watch and saying she was hungry!"
Once again, the family was impressed with the level of care their daughter received from Liver Care Center staff. They were particularly impressed with the way the entire health care team made a point of including Dominique in discussions of her condition. "During rounds Maria Alonso, MD, spoke directly to her and the residents answered Dominique's questions and made a point of listening to her," says Drunette. Fred Ryckman, MD, director of liver transplant, who had performed Dominque's original Kasai procedure, did the liver transplant alongside Dr. Alonso co-surgical director. "Many members of our care team have been with us since Dominique was first diagnosed. They are like family to us," says Drunette.
Dominique was hospitalized for just nine days following her liver transplant. She could not return to school for a minimum of 90 days and the family was advised to keep her primarily at home in order to protect her immune system and keep her complication-free. For Dominique, a "social butterfly," according to her mom, the isolation was perhaps the most challenging part of the entire process. "She counted down the days to when she could go back to the mall and hang around her friends and family."
Looking Ahead
In many ways Dominique is a typical 13-year old: she loves to talk to friends on the phone, play board games and decorate cakes with her mom. But unlike her peers, Dominique has been forced to live with a life-threatening disease. It's a challenge that she and her family have met head-on. If her history with the disease is any indication, bililary atresia is a test she'll pass with flying colors.
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